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Primer removal during mammalian mitochondrial DNA replication

Review article
Authors Jay Uhler
Maria Falkenberg
Published in DNA Repair
Volume 34
Pages 28-38
ISSN 1568-7864
Publication year 2015
Published at Institute of Biomedicine, Department of Medical Biochemistry and Cell Biology
Pages 28-38
Language en
Links dx.doi.org/10.1016/j.dnarep.2015.07...
Keywords mtDNA, RNA primer, RNase H1, FEN1, DNA2, MGME1, okazaki fragment maturation, base excision-repair, polymerase-gamma, ligase-iii, ribonuclease-h, in-vitro, transcription termination, subcellular-localization, embryonic lethality, strand displacement, Genetics & Heredity, Toxicology
Subject categories Genetics

Abstract

The small circular mitochondrial genome in mammalian cells is replicated by a dedicated replisome, defects in which can cause mitochondrial disease in humans. A fundamental step in mitochondrial DNA (mtDNA) replication and maintenance is the removal of the RNA primers needed for replication initiation. The nucleases RNase H1, PEN1, DNA2, and MGME1 have been implicated in this process. Here we review the role of these nucleases in the light of primer removal pathways in mitochondria, highlight associations with disease, as well as consider the implications for mtDNA replication initiation. (C) 2015 The Authors. Published by Elsevier B.V. This is an open access article under the CC BY-NC-ND license.

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